期刊
MOLECULAR SYNDROMOLOGY
卷 5, 期 3-4, 页码 187-197出版社
KARGER
DOI: 10.1159/000360101
关键词
Clinical indications; Coenzyme Q(10); Coenzyme Q(10)-related; compounds; Pharmacokinetics
资金
- Ministerio de Sanidad, Spain [FIS PI10/00543, FIS EC08/00076]
- Fondo Europeo de Desarrollo Regional (FEDER-Union Europea)
- Servicio Andaluz de Salud-Junta de Andalucia [SAS 111242]
- Proyecto de Investigacion de Excelencia de la Junta de Andalucia [CTS5725]
- AEPMI (Asociacion de Enfermos de Patologia Mitocondrial)
- FEEL (Fundacion Espanola de Enfermedades Lisosomales)
- ALBA Andalucia (Federacion Andaluza de Fibromialgia y Fatiga Cronica)
For a number of years, coenzyme Q(10) (CoQ(10)) was known for its key role in mitochondrial bioenergetics; later studies demonstrated its presence in other subcellular fractions and in blood plasma, and extensively investigated its antioxidant role. These 2 functions constitute the basis for supporting the clinical use of CoQ(10). Also, at the inner mitochondrial membrane level, CoQ(10) is recognized as an obligatory cofactor for the function of uncoupling proteins and a modulator of the mitochondrial transition pore. Furthermore, recent data indicate that CoQ(10) affects the expression of genes involved in human cell signaling, metabolism and transport, and some of the effects of CoQ(10) supplementation may be due to this property. CoQ(10) deficiencies are due to autosomal recessive mutations, mitochondrial diseases, aging-related oxidative stress and carcinogenesis processes, and also statin treatment. Many neurodegenerative disorders, diabetes, cancer, and muscular and cardiovascular diseases have been associated with low CoQ(10) levels as well as different ataxias and encephalomyopathies. CoQ(10) treatment does not cause serious adverse effects in humans and new formulations have been developed that increase CoQ(10) absorption and tissue distribution. Oral administration of CoQ(10) is a frequent antioxidant strategy in many diseases that may provide a significant symptomatic benefit. (C) 2014 S. Karger AG, Basel
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